What is Acne Fulminans?
Acne fulminans, also known as acne maligna, is a rare and severe form of acne that can cause pain, open sores, and bleeding. It may come with other symptoms like fever and joint pain. There could also be changes in your bones and abnormal results from lab tests. Some people may mistake it for another type of severe acne, called acne conglobata. Acne fulminans is typically hard to treat with regular acne antibiotics.
What Causes Acne Fulminans?
Acne fulminans is a rare skin condition that experts still don’t fully understand. What we do know is that it can be triggered by using high doses of an acne medication called isotretinoin, particularly when first starting the treatment for severe cases of acne. It also seems to be somehow related to increased testosterone levels.
For instance, anabolic steroids, which increase the levels of a beneficial but oily substance on our skin called sebum, could contribute to the development of acne fulminans. Anabolic steroids are linked to a rise in a type of skin bacteria called Propionibacterium acnes. This is why bodybuilders who use anabolic steroids often develop this form of acne very quickly. Some believe that the increase of this skin bacteria or antigens similar to it prompts the immune system to react, resulting in acne fulminans.
Others speculate that the condition is due to an autoimmune response. This theory comes from the fact that some people with Acne fulminans have been found to have circulating immune complexes, a sign that the body’s immune system is overactive.
Acne fulminans also seems to have a genetic component, although we don’t yet know exactly how it is inherited. Certain syndromes appear to be related to this skin condition, including SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome, PAPA (pyogenic arthritis, pyoderma gangrenosum, and acne) syndrome, PASH (pyoderma gangrenosum, acne, and hidradenitis suppurativa) syndrome, and PAPASH (pyogenic arthritis, pyoderma gangrenosum, acne, and hidradenitis suppurativa) syndrome.
Risk Factors and Frequency for Acne Fulminans
Less than 200 cases of a specific disorder have been reported so far. This disorder mostly affects young white males between the ages of 13 and 22, and these young people usually have a history of acne. Though less common, there have also been some cases reported in females. The disorder has been reported worldwide, but the number of new cases seems to be decreasing.
- Less than 200 cases of this disorder have been reported.
- It typically affects young white males between 13 to 22 years old.
- Most affected individuals have a history of acne.
- There are a few cases reported in females.
- The disease has been reported around the world, but it seems to be on a decline.
Signs and Symptoms of Acne Fulminans
Acne fulminans is a severe form of acne that comes on very suddenly. This kind of acne often results in ulcers, and patients usually experience fever and pain in several joints. It’s common for patients to mention that standard acne treatments haven’t been successful for them, and they usually have a history of acne.
One can compare acne fulminans to another type of severe acne known as acne conglobata, as both involve many inflamed lumps on the body. These lumps can become quite painful, may turn into ulcers, bleed, and be covered with a crust. However, there’s a key difference between the two; in acne fulminans, there aren’t polyporous comedones, which are a type of acne spot that’s large and filled with pus. Acne fulminans may also cause an enlarged and painful spleen, nodules on the skin, and other symptoms affecting the whole body.
Those suffering from acne fulminans may also show symptoms that extend beyond skin issues. If systemic symptoms exist, they may have a hunched position due to pain in bones and joints. Joint pain associated with this condition can affect multiple joints, usually in the knees, hips, and pelvic region.
Testing for Acne Fulminans
If a doctor suspects that a patient has a particular disorder, they may arrange for certain tests to be carried out as part of the examination. A complete blood count might be scheduled, which could show different signs like an elevated white blood cell count. This test might also detect anemia, which is another indicator of the disease. Additionally, tests such as the erythrocyte sedimentation rate and C-reactive protein tests might be undertaken, as these could be higher in patients with this disorder. Other tests could include a liver function test and, in females, a test for human chorionic gonadotrophin levels in blood or urine.
Because patients often experience bone and joint pain, imaging usually forms part of the workup for this disorder. In its early phases, X-rays may only show certain reactions in the body. Around half of patients may show destructive lesions on plain X-ray film. A bone scan might also highlight several areas where the body has reacted with increased uptake. These lesions often look like symptoms of acute osteomyelitis. Despite this, these areas often test negative in cultures.
Treatment Options for Acne Fulminans
The recommended treatment for severe acne, known as acne fulminans, involves a combination of corticosteroids and a medication called isotretinoin. Oral corticosteroids are typically started first at high doses and taken for at least two weeks until the acne lesions start to heal. Following this, treatment with isotretinoin can begin. It’s initially given at a low dose, alongside continued corticosteroid treatment. If after four weeks there’s no worsening of the acne, this same dose of isotretinoin is maintained for another four weeks. The dose of corticosteroids is then gradually reduced whilst continuing with isotretinoin.
Treatment with isotretinoin is usually required for several months, as a low starting dose is used. If the acne does come back after isotretinoin is stopped, a repeated course at a higher dose may be recommended.
Medical research suggests that also using strong topical corticosteroids alongside these medications can lead to a quicker improvement in acne. As the acne starts to heal, symptoms reduce and acne spots become smaller and less sore. Scarring is, however, common with this type of acne.
Traditional antibiotics that are typically used to treat standard acne aren’t effective for acne fulminans. In cases where acne fulminans is resistant to other treatments, biologic drugs such as etanercept and infliximab have shown promising results, although it’s still unknown whether these drugs will work for everyone.
Other treatments that have been used with moderate success include a type of laser treatment called pulsed dye laser. However, side effects and pain can occur, and large spots may require multiple treatments. Other medications, such as diaminodiphenyl sulfone, prednisone combined with dapsone or cyclosporine, have been used successfully in some cases.
Patients with acne fulminans are ideally managed by both a dermatologist and an internal medicine doctor. It’s important to note that women of childbearing age must be very careful when using retinoids, as these medications can cause birth defects. Women are advised to avoid becoming pregnant for at least one month after stopping isotretinoin.
What else can Acne Fulminans be?
- PASH syndrome – a condition that involves acne and a skin disorder known as suppurative hidradenitis
- Acne conglobata – a severe form of acne that causes deep and painful abscesses on the skin
- Acne vulgaris – the most common type of acne, often thought of as ‘teenage acne’
- Bromoderma – a skin condition caused by excessive bromine exposure
- Folliculitis decalvans – a rare skin disorder that primarily causes hair loss and scarring
- Iododerma – a skin reaction that occurs due to excessive consumption or application of iodine
- Rosacea fulminans – a sudden severe onset of rosacea, a skin condition that causes redness and visible blood vessels
- Severe cystic acne – a type of acne which causes large, red, and painful breakouts deep in your skin
All of these are different skin disorders that can sometimes be mistaken for each other due to their similar symptoms. However, each requires a different treatment approach.
